Wetter, David A.

ABSTRACTBackgroundDrug‐induced hypersensitivity syndrome (DiHS), or drug reaction with eosinophilia and systemic symptoms (DRESS), is a delayed and severe immune response to certain medications. We investigated vancomycin‐induced DiHS/DRESS, notable for frequent and severe organ involvement, to describe its specific histopathology and the correlations between histopathologic and clinical findings.MethodsA retrospective review was conducted to identify patients between 2006 and 2022 who received vancomycin, had archived skin biopsy specimens, and were scored as having probable or definite DiHS/DRESS. Clinical features were retrospectively collected, and biopsy specimens were reviewed by a board‐certified dermatopathologist. A subset of histopathologic and clinical features was analyzed for statistical correlation.ResultsTwenty‐three patients met the inclusion criteria. Most biopsy specimens (87%) showed an eczematous reaction pattern; 17 (74%) showed a secondary reaction pattern. Spongiosis (87%) and neutrophilic infiltration (91%) were common epidermal characteristics. The dermal inflammatory infiltrate was frequently superficial (87%) and consistently included plasma cells (96%). Eosinophils were present in the dermis in 70% of cases. Parakeratosis negatively correlated with liver involvement and positively correlated with desquamative rash. Epidermal lymphocytes were negatively correlated with the RegiSCAR score.ConclusionsVancomycin‐associated DiHS/DRESS histopathology was characterized by a frequent eczematous reaction pattern, multiple coexisting reaction patterns, and epidermal neutrophilic infiltration.

Abstract Background Perianal draining tunnels in hidradenitis suppurativa (HS) and perianal fistulizing inflammatory bowel disease (IBD) present diagnostic and management dilemmas. Methods We conducted a retrospective chart review of patients with perianal disease evaluated at Mayo Clinic from January 1, 1998, through July 31, 2021. Patients' demographic and clinical data were extracted, and 28 clinical features were collected. After experimenting with several machine learning techniques, random forests were used to select the 15 most important clinical features to construct the diagnostic prediction model to distinguish perianal HS from fistulizing perianal IBD. Results A total of 263 patients were included (98 with HS, 100 with IBD, and 65 with both IBD and HS). Patients with HS had a higher mean body mass index, a higher smoking rate, and more commonly showed cutaneous manifestations of tunnels and comedones, while fistulas, abscesses, induration, anal tags, ulcers, and anal fissures were more common in patients with IBD. In addition to having lesions in the perianal area, patients with IBD often had lesions in the buttocks and perineum, while those with HS had additional lesions in the axillae and groin. Among the statistically significant features, the 15 most important were identified by random forest: fistula , tunnel , digestive symptom , knife‐cut ulcer , perineum , body mass index , age , axilla , abscess , tags , smoking , groin , genital cutaneous edema , erythema , and bilateral/unilateral . Conclusions The results of this study may help differentiate perianal lesions, especially perineal HS and fistulizing perineal IBD, and provide promise for a better therapeutic outcome.

AbstractBackgroundThere is a dearth of studies investigating the efficacy of hydroxychloroquine in the treatment of either anogenital lichen sclerosus or extragenital lichen sclerosus, a condition that, if left untreated, could lead to a greater degree of scarring and malignant transformation.ObjectiveThis study aims to analyze the demographic characteristics, clinicopathological features, treatment response, and outcomes of patients diagnosed with either anogenital or extragenital lichen sclerosus who received hydroxychloroquine therapy.MethodsA retrospective analysis was conducted involving 70 patients diagnosed with lichen sclerosus who underwent treatment with hydroxychloroquine at our institution between 2018 and 2023.ResultsAmong the cohort, 67 patients were female, and 3 were male. Extragenital lichen sclerosus was diagnosed in 23 patients, with 16 exhibiting concomitant morphea overlap. Itching was the predominant clinical presentation (67%). A notable proportion of patients (36%) had a connective tissue disorder, prompting hydroxychloroquine therapy. Among the 30 patients treated solely for lichen sclerosus, 21 demonstrated response and 9 had no response. From a broader comparison of response to hydroxychloroquine, the overall anogenital response rate was 84.6% as opposed to 50% in extragenital lichen sclerosus. The median time to initial response was 4 months. Adverse effects, predominantly mild, were observed in 10 (14.3%) patients.LimitationThis study is constrained by its retrospective nature and reliance on data from a single center, resulting in a limited sample size.ConclusionHydroxychloroquine demonstrates promise as a therapeutic option for anogenital lichen sclerosus because of its favorable response rates and low incidence of adverse effects. However, further investigations, including larger‐scale or prospective studies, are imperative to ascertain its definitive efficacy.

Abstract Background We aimed to investigate the prevalence of skin disease among patients with systemic lupus erythematosus (SLE) and determine whether LE skin disease had clinical or serologic correlates with SLE. Methods We reviewed records of 335 patients with SLE (seen at Mayo Clinic, Rochester, Minnesota, USA) and abstracted skin manifestations, fulfilled mucocutaneous SLE criteria, and clinical and serologic parameters. Results Of the 231 patients with skin manifestations, 57 (24.7%) had LE‐specific conditions, 102 (44.2%) had LE‐nonspecific conditions, and 72 (31.2%) had both. LE skin disease was associated with photosensitivity, anti‐Smith antibodies, and anti‐U1RNP antibodies (all P < 0.001). Patients without LE skin disease more commonly had elevated C‐reactive protein levels ( P = 0.01). Patients meeting 2–4 mucocutaneous American College of Rheumatology criteria less commonly had cytopenia ( P = 0.004) or anti−double‐stranded DNA antibodies ( P = 0.004). No significant associations were observed for systemic involvement (renal, hematologic, neurologic, and arthritis) when comparing patients with or without LE skin involvement. LE skin involvement was not significantly associated with internal SLE disease flare, number of medications, or overall survival. Conclusions LE skin disease commonly occurs in patients with SLE. The presence of LE skin disease had no mitigating impact on the severity of SLE sequelae, disease flares, number of medications, or overall survival.

AbstractBackgroundDermatology grand rounds are popular educational activities among dermatologists and trainees, contributing to improved knowledge and skills. Dermatology hospital conferences at Mayo Clinic Dermatology are separate from dermatology grand rounds in that they focus on inpatient consultations and dermatology admissions.ObjectivesThis study aimed to determine the types of cases presented during dermatology hospital grand rounds and evaluate the educational value of this continuing medical education (CME)‐accredited activity.MethodsThis retrospective study reviewed all inpatient dermatology cases presented during hospital grand rounds at Mayo Clinic Dermatology in Rochester, MN from 2019 to 2022; this study also reviewed the compiled surveys of faculty participants at our institution from 2019 to 2022.ResultsA total of 389 cases met the inclusion criteria for analysis. The most common diagnostic category was inflammatory/autoimmune diseases (157 cases, 40%), followed by infection or infestation (73 cases, 19%), drug‐induced eruptions (49 cases, 13%), neoplastic or metastatic diseases (41 cases, 11%), genetic/congenital disorders (21 cases, 5%), depositional/nutritional/metabolic conditions (15 cases, 4%) and wound/traumatic/iatrogenic dermatoses (12 cases, 3%), which were the least common. Most cases were presented for discussion of diagnosis and management (246 cases, 63%), but others focused on the arrival of diagnosis (119 cases, 31%) or management alone (24 cases, 6%). The compiled surveys of faculty participants (n = 50) indicated high satisfaction with dermatology hospital grand rounds, with the majority expressing that evidence‐based research was incorporated and positively impacted their practice. Most respondents indicated that hospital conference participation improved knowledge (97%), competence (64%), clinician performance (54%), patient outcomes (52%) and team performance (50%).ConclusionsThe cases presented during these conferences spanned from challenging inflammatory diseases to simple dermatoses, with over 800 literature references reviewed. Dermatology hospital grand rounds provide high value as a CME‐accredited activity for dermatology faculty, even for those without a specific interest in hospital/inpatient dermatology.

AbstractIgA vasculitis is a small‐vessel vasculitis subtype with increased risk of systemic involvement. We aimed to investigate if any light‐microscopic features can predict the presence of perivascular granular IgA deposits on direct immunofluorescence (DIF) microscopy. We performed a retrospective search of cutaneous pathology reports from our internal and consultation practice (January 1, 2010–October 5, 2021) with a diagnosis of leukocytoclastic vasculitis and accompanying DIF. A blinded dermatopathologist reviewed standard microscopy slides for predetermined histopathological features. Fifty‐six biopsies (48 patients) and 56 biopsies (42 patients) met inclusion criteria for IgA+ and IgA−, respectively. The presence of eosinophils and mid and deep dermal inflammation were statistically more associated with IgA− (41/56 [73.2%] and 31/56 [55.4%], respectively) than IgA+ cases (28/56 [50.0%] and 14/56 [25.0%]; p = 0.049 and 0.006, respectively, chi‐squared test). Other microscopic criteria recorded were not significantly different between the two groups (p > 0.05, chi‐squared and Fisher's exact tests). In this retrospective study of 112 cases, we found that while the absence of eosinophils and absence of mid‐ and deep inflammation were correlated with increased likelihood of IgA perivascular deposition on DIF, no other histopathological features on light microscopy tested could reliably predict the presence of IgA perivascular deposition on DIF. Therefore, DIF remains a necessary component for the accurate diagnosis of cutaneous IgA vasculitis.

Abstract Background Reactive granulomatous dermatitis (RGD) is an umbrella term used to describe interstitial granulomatous dermatitis (IGD), palisaded neutrophilic and granulomatous dermatitis (PNGD), and interstitial granulomatous drug eruption (IGDR). Objective The aim of this study was to describe systemic associations of RGD, explore possible associations between histopathologic findings and systemic RGD associations and determine clinical relevance of RGD subtypes. Methods We retrospectively studied clinical and histopathologic characteristics of patients with RGD from 1990 through 2020. Results Of 65 patients with RGD (41 women, 24 men; median age at diagnosis, 62 years), 37 had IGD, 26 had PNGD, and 2 had IGDR. Fifty patients (76.9%) had an associated systemic condition; rheumatologic conditions were identified for 34 (52.3%) patients. The associated systemic condition occurred before RGD in approximately 75% of patients. Statistical analyses did not show significant associations between specific subtypes of RGD and systemic diseases or treatment response, and specific histopathologic findings were not predictive of an associated systemic disease. Conclusions Although most patients with RGD had an associated systemic condition, subtypes of RGD did not correlate with systemic associations, lending support to the use of the umbrella term RGD .

AbstractBackgroundUnderstanding whether specific histopathologic features on skin biopsy are predictive of systemic associations in dermatomyositis (DM) would be useful to guide clinical screening.MethodsThrough retrospective medical record search, clinical and laboratory findings of patients with DM were documented. Existing skin biopsy slides were re‐reviewed blindly.ResultsOf all biopsy specimens (n = 42), the most frequent histopathological finding was vacuolar interface dermatitis (95%). Other features included perivascular lymphocytic infiltrate (71%), increased dermal mucin (40%), vessel wall thickening (12%), follicular plugging (9.5%), and dermal sclerosis (7%). Neutrophilic infiltrate was observed in three biopsies from a patient with adalimumab‐associated DM. Vasculitis was not observed. There was no statistically significant difference in the presence of any histopathological feature and that of various systemic manifestations (i.e., myopathy, interstitial lung disease [ILD] and malignancy). However, we observed that dense lichenoid infiltrate rather than pauci‐inflammatory changes correlated with severe itching (p < 0.001). Patients with MDA‐5 antibodies were significantly more likely to have vasculopathy than those without (p = 0.029*).ConclusionsNo dermatopathologic feature was reliably predictive of myopathy, ILD, or malignancy. This finding implies that, regardless of histopathologic findings, patients should be screened for associated conditions as clinically indicated.